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Potent and Selective Activity-Based Probes for GH27 Human Retaining α-Galactosidases.
- Source :
-
Journal of the American Chemical Society . 8/20/2014, Vol. 136 Issue 33, p11622-11625. 4p. 2 Black and White Photographs, 1 Graph. - Publication Year :
- 2014
-
Abstract
- Lysosomal degradation of glycosphingolipids is mediated by the consecutive action of several glycosidases. Malfunctioning of one of these hydrolases can lead to a lysosomal storage disorder such as Fabry disease, which is caused by a deficiency in a-galactosidase A Herein we describe the development of potent and selective activity-based probes that target retaining α-galactosidases. The fluorescently labeled aziridine-based probes 3 and 4 inhibit the two human retaining α-galactosidases αGal A and αGal B covalently and with high affinity. Moreover, they enable the visualization of the endogenous activity of both a-galactosidases in cell extracts, thereby providing a means to study the presence and location of active enzyme levels in different cell types, such as healthy cells versus those derived from Fabry patients. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 00027863
- Volume :
- 136
- Issue :
- 33
- Database :
- Academic Search Index
- Journal :
- Journal of the American Chemical Society
- Publication Type :
- Academic Journal
- Accession number :
- 98249927
- Full Text :
- https://doi.org/10.1021/ja507040n